Benign Tumours and Pseudotumours Within the Porta Hepatis Masquerading as Perihilar Cholangiocarcinoma

Abstract

Background: Hilar cholangiocarcinoma (HC), also referred to as Altemeier-Klatskin's tumour, is a lethal primary extrahepatic carcinoma of biliary epithelial origin, aris ing within 2 cm of the hilar confluence. Radical surgical excision provides the best chance for a cure; however, the management of patients with HC is challeng ing not only because of the need for a high level of skill in biliary and hepatic resections, but also because of the difficulty in reach ing an accurate dia gnosis preoperatively. In fact, the differential dia gnosis of HC is a dia gnostic dilemma which is currently persisting, as modern, sophisticated dia gnostic modalities are not always able to provide a definitive preoperative dia gnosis. This difficulty is compounded by the fact that alternative entities that mimic HC may be present in up to 25% of patients with hilar obstruction. This makes precise preoperative characterisation of a hilar stricture extremely important by prevent ing unnecessary, high-risk, major surgical procedures. Therefore, alternative benign entities masquerad ing as Altemeier-Klatskin's tumour deserve an important place in the differential dia gnosis of hilar obstruction. Purpose: Consider ing the important clinical implications that a precise dia gnosis of the aetiological cause of a biliary obstruction at the liver hilum would have, this paper will focus on the differentiation between HC and benign hilar obstructions and will review benign tumours and pseudotumours masquerad ing as HC along with their specific dia gnostic features.